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world cancer report - iarc

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LYMPHOMA<br />

SUMMARY<br />

> Malignant lymphomas are classified as<br />

either Hodgkin disease or non-Hodgkin<br />

lymphoma.<br />

> Hodgkin disease afflicts mainly children<br />

and the elderly in developing countries<br />

and young adults in more developed<br />

countries; 62,000 new cases are diagnosed<br />

annually.<br />

> The incidence of malignant non-Hodgkin<br />

lymphomas is increasing <strong>world</strong>wide;<br />

more than 280,000 new cases occur<br />

annually, predominantly in more developed<br />

countries.<br />

> Burkitt lymphoma is a subtype of malignant<br />

B-cell lymphoma common in Africa<br />

in regions with endemic malaria. B-cell<br />

lymphomas may also be caused by<br />

immunosuppression. Both are commonly<br />

associated with the Epstein-Barr virus.<br />

> Advances in chemotherapy have led to a<br />

five-year survival rate for Hodgkin disease<br />

of more than 70% and that for non-<br />

Hodgkin lymphomas has increased to<br />

60-70%.<br />

Definition<br />

The term lymphoma covers a heterogeneous<br />

group of neoplasms of lymphoid tissue.<br />

Traditionally, lymphomas are categorized<br />

as either Hodgkin disease or non-<br />

Hodgkin lymphoma, these distinct entities<br />

having different patterns of behaviour and<br />

response to treatment. Within each of<br />

these two entities there is a range of<br />

diverse subtypes.<br />

Epidemiology<br />

Non-Hodgkin lymphomas are a very varied<br />

group of neoplasms. Excluding the types<br />

that generally manifest as leukaemias<br />

rather than single or multiple aggregates<br />

of cells, there are around 287,000 cases<br />

of non-Hodgkin lymphoma in the <strong>world</strong><br />

< 3.7<br />

< 5.0<br />

Fig. 5.97 Global incidence of non-Hodgkin lymphoma in men. The disease is most common in developed<br />

countries, although there are areas of moderate to high incidence in some Middle-Eastern countries and<br />

in parts of sub-Saharan Africa.<br />

per year. More males than females are<br />

affected (17.1 cases per 100,000 males<br />

compared to 11.2 cases per 100,000<br />

females in the USA) and incidence<br />

increases with age. Geographically, non-<br />

Hodgkin lymphoma is most common in<br />

developed countries (52% of the <strong>world</strong><br />

total cases, and the seventh most common<br />

<strong>cancer</strong> in more developed countries),<br />

although in the developing <strong>world</strong> there are<br />

areas of moderate to high incidence in<br />

some Middle-Eastern countries (Saudi<br />

Arabia, Israel) and in parts of sub-Saharan<br />

Africa (Fig. 5.97). The latter is due to the<br />

high incidence of Burkitt lymphoma, an<br />

aggressive subtype of non-Hodgkin<br />

lymphoma, particularly in children in tropical<br />

Africa. Papua New Guinea also has<br />

high rates of Burkitt lymphoma.<br />

The incidence rates of non-Hodgkin lymphoma<br />

have risen dramatically in the last<br />

20 years, particularly in developed countries,<br />

including Western Europe, North<br />

America and Australia (Fig. 5.99). This<br />

may in part reflect better diagnosis, or<br />

< 6.8<br />

< 8.9<br />

Age-standardized incidence/100,000 population<br />

< 16.1<br />

changing classification systems. However,<br />

these considerations together do not<br />

account for the extent of increase.<br />

Likewise, the fact that non-Hodgkin lymphoma<br />

is a complication of AIDS (occurring<br />

in up to 5-10% of AIDS cases in developed<br />

countries) does not completely<br />

account for the increasing trend. In contrast<br />

to incidence, mortality rates have, in<br />

general, been declining as a consequence<br />

of improvement in therapy.<br />

Hodgkin disease comprises about 23% of<br />

malignant lymphomas <strong>world</strong>wide (about<br />

62,000 annual cases). There is a male predominance<br />

(sex ratio 1.6:1). In developing<br />

countries, Hodgkin disease (predominantly<br />

the mixed cellularity subtype) occurs<br />

mainly in children and in the elderly, while<br />

in developed countries there is a peak in<br />

young adults (mainly the nodular sclerosing<br />

subtype). The disease is rare in<br />

Eastern and South-Eastern Asian populations<br />

at any age (Fig. 5.100). The pattern<br />

of Hodgkin disease in black Americans<br />

more closely resembles that of white<br />

Lymphoma<br />

237

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